Objective <p>The aim of the current study was to describe neuroimaging findings of Epstein–Barr virus (EBV)-associated primary central nervous system lymphoproliferative disorder (PCNS-LPD) in human immunodeficiency virus (HIV)-positive individuals.</p> Methods <p>The clinical data of four HIV-positive patients with confirmed EBV-associated PCNS-LPD were retrospectively analyzed from November 2018 to August 2024. All patients had undergone magnetic resonance imaging (MRI) examinations. The location, signal intensity, and enhancement pattern of the lesions on MRI were retrospectively evaluated.</p> Results <p>The pathological diagnosis of EBV-associated PCNS-LPD was made in all four patients, in two cases by surgery and in two by biopsy. All four patients presented with limb motor dysfunction, and three had dizziness or headache. On MRI, lesions were located in the periventricular white matter in three patients; they involved the left cerebral peduncle and basal ganglia in two patients, the medulla oblongata in two patients, the right cerebellar peduncle and splenium of the corpus callosum in one patient, and the cortex and subcortex in one patient. All lesions showed hypointense to isointense signal intensity (SI) on T1-weighted imaging and isointense to hyperintense SI on T2-weighted imaging, as well as exhibiting restricted diffusion in the peripheral portions of the lesions. All lesions showed either ring-like or nodular inhomogeneous enhancement.</p> Conclusion <p>In patients with HIV/acquired immune deficiency syndrome (AIDS) and EBV infection, multiple lesions in the periventricular regions and other intracranial sites should raise suspicion for EBV-associated PCNS-LPD. Although imaging findings may provide some evidence of EBV-associated PCNS-LPD, a differential diagnosis remains necessary.</p>